Madeline's JDM Story: May 2008

Sunday, May 25, 2008

Swollen face

Madeline woke up this morning and I noticed her face was slightly swollen. Was it the sun, heat, meds??? I then realized I tried the new spf 85 neutrogena sunblock on her yesterday. Won't use that again. We stayed indoors today and will not put any sunblock on. I want to give her little face a rest.

Saturday, May 24, 2008

Heat

Madeline went with me to Trey's baseball game. She really complained about the heat and her face was terrible. She almost could not stand to be out there. This is not usual for her.

Thursday, May 22, 2008

Oh my gosh - she has a ringworn

Madeline came to work with me today. One, because she wanted to. Two, I wanted the nurses to see her neck. And yes, she has a ringworm - UGH!

I called our pedi and got the "cream". Where in the world did she get that one???

Madeline has tryouts tonight for her dance company - competition dance team. She is excited about that!

Wednesday, May 21, 2008

Best day since taking methotraxate

Well, we took her metho last night and today has been the best day since she started her meds. Maybe her little body is getting better with the drugs.

Tuesday, May 20, 2008

Last day of school

Trey graduates from the 6th grade today. It's hard to believe he is going to Jr. High - Lord help everyone. Madeline and Trey will not be at the same school again until Trey is a senior in high school and she is a freshman - kind of sad.

Rick and I somehow got tagged to plan Trey's 6th grade dance after his graduation (which again I had to bite my lip not to cry). Madeline was so excited to help so she could spy on the big kids. They all had a good time and we went home to enjoy the start of summer.

We are so lucky to have such wonderful kids. We are blessed.

Monday, May 19, 2008

Last full day of school

Today is awards day for Madeline. She looks so good today and her mood is wonderful. Rick and I went to her awards ceremony and I had to bite my tongue to not cry. We are so proud of her. She won six awards (I think the most in her class). She is an exceptional, beautiful girl.

Sunday, May 18, 2008

Lafayette weekend

We are so lucky to have two of the most beautiful and talented children. Madeline's brother, Trey (who is 12) is a wonderful baseball player and plays on a travel team that his dad is the coach. With travel ball, we TRAVEL. So off to Lafayette we went for a tournament. My best friend and Madeline's godmother lives in Lafayette so we planned some "girl time" together since Trey's first game was at 1 p.m. in the heat of the day.

We shopped and shopped, but best of all Madeline got to ride Aunt Holly's horse, Dalmatian. She loved it.

Trey and Rick did well in the tournament, but we were home early Sunday afternoon meaning we lost after three games. Oh well...we had fun!

Friday, May 16, 2008

Field Day at School

I was dreading, dreading, dreading field day today. Madeline's dad and I both have busy days at work and could not go to field day, therefore not able to make sure she wears sunblock, hat, sunglasses, etc. Plus, they stay outside for four or more hours.

But luckily...it rained. YEA! She was a little disappointed, but they got to play games inside. All is good.

Thursday, May 15, 2008

May 15

Madeline has dance on Thursdays. She woke up in a good mood, but her face seemed redder today. I can't keep up with her rash. It gets better, than two days later it's terrible again. I don't get it at all.

She did mention something odd to me. She said she does not like tap class for how it makes her feet feel - the vibrations and the noise. She said it almost makes her feet hurt. We will have to watch this.

Wednesday, May 14, 2008

May 14

We took the methotraxate last night and I anticipated the possessed child today, but she seemed to tolerate the meds better. She was still somewhat tired though.

Tuesday, May 13, 2008

May 12 & 13

Good days, about the same. Face seems a little better, but her chest rash is what has improved the most. Her knees seem to be improving too. Good days.

Sunday, May 11, 2008

May 11

After that long busy day yesterday, Madeline swam and jumped on the trampoline all day. We loaded her with sunblock and waited until later in the day to go out. But she tolerated all well. Her face looks better today.

Saturday, May 10, 2008

Dance Recital Day

Madeline dance in five recitals today - she and I were both wore out! She did beautifully. She does not seem as limber as she once was - don't know if is disease or meds or both. Her dad and I were so proud of her. She got an enormous trophy and her daddy gave her a bouquet of flowers - which Madeline loves. Even her brother was somewhat nice to her today. :)

Friday, May 9, 2008

May 8

Madeline's dance recital is coming up this weekend. She has dress rehearsal on Friday from 4 p.m until 9 p.m. I hope she can handle and stay up with the other girls. She is excited so that should help. She does seem better today and her hand blisters are not as bad. They are not 100 percent gone, but probably 50 percent improved.

Face is still red.

Wednesday, May 7, 2008

May 7

Madeline has a lot of stomach aches lately. I guess it is the medicine. In fact, Rick had to pick her up from school today due to the pain. I think her little body is going to have to start all over with getting use to the drugs. I am so thankful school is almost out. I think we all need the rest from worrying about her getting sick, not putting on sunblock for recess/PE, children picking on her.

Tuesday, May 6, 2008

May 6

Madeline is of course - tired. It was a busy day yesterday though. Her face looks a little better. We will take the cyclo and metho tonight. Back to our old habit of medications.

Monday, May 5, 2008

May 5 - Field Trip Day

Madeline is very excited about her 3rd grade class field trip. Both Rick and I are going with her. One thing this has taught us is that every day is precious and she will not be little forever. We don't want to miss a thing. We went to the Tabasco plant at Avery Island and the Lafayette Zoo - it was a good day.

The doctor wants us to start the cyclosporine again, so we will tonight.

Saturday, May 3, 2008

May 3

I had Madeline rest a lot this weekend. Her rash and blisters are terrible - of course. Something new is going on though - she had a bloody nose. She in her 9 years of life has never had a bloody nose. Odd.

Friday, May 2, 2008

May 2

Madeline is very red, hands still have sores and hurt her badly. I called the pediatrician and he said he would call ped. rheum. on Monday. We still are off meds and she is "MOODY"!

Thursday, May 1, 2008

May 1

Madeline's hands are still sore and red. She is in a better mood. Maybe she is getting well with this "secondary illness". I guess I am a doctor now...LOL.

She is still off her meds.

Blood Work Results

May 25:SGOT (AST) 24 (0-31, normal range); SGPT (ALT) - 21 (0-31, normal range); CPK (CK) - 111 (24-170, normal range);LDH - 189 (120-300, normal range); Aldolase - 6.2 (3.3-9.7, normal range)
Feb. 26:SGOT (AST) 27 (0-31, normal range); SGPT (ALT) - 21 (0-31, normal range); CPK (CK) - 159 (24-170, normal range);LDH - 170 (120-300, normal range); Aldolase - 4.1 (3.3-9.7, normal range)
Dec. 31:SGOT (AST) 25 (0-31, normal range); SGPT (ALT) - 21 (0-31, normal range); CPK (CK) - 98 (24-170, normal range);LDH - 187 (120-300, normal range); Aldolase - 6.3 (3.3-9.7, normal range)
Aug. 27: ALT - 10 (normal range 0-31); AST - 22 (0-31, normal); CPK - 125 (24-170, normal); LDH - 211 (120-300, normal); Aldolase - 4.6 (3.3-9.7, normal)
June 15: ALT - 11 (normal range 0 -31); AST - 25 (normal range, 0-31); CPK - 112 (normal range, 24-170); LDH - 225 (normal range, 120-300); Aldolase - 5.2 (normal range 3.3- 9.7)
March 20: ALT 11 (normal range 0 - 31); AST 24 (normal range 0 - 31)
Dec. 29 - ALT 10 (normal range 0 - 31); AST 20 (normal range 0 - 31)
Oct. 24 - ALT 10 (normal range 0 - 31); AST 24 (normal range 0 - 31)
Aug. 22 - Cyclsporine A Serum leve: 97.5; ALT 9 (normal range 0-31); AST 22 (normal range 0 - 31); urinalysis - all clear
July 14 - Cyclosporine A Serum level: 141.0
June 27: ALT 11 (normal range 0 - 31); AST 24 (normal range 0 - 31)

What is JDM?

Source curejm.com
Juvenile Myositis (JM), including Juvenile Dermatomyositis (JDM), Amyopathic Dermatomyositis (AMD) and Juvenile Polymyositis (JPM), is an autoimmune disease affecting approximately 5,000 children in the United States. Weak muscles and skin rash are the primary symptoms of JDM, while muscle weakness without a rash is the primary symptom of JPM. Children afflicted with AMD, on the other hand, experience the skin rash without the weakened muscles. Even within these three designations, JM affects every child differently. Some children experience a mild form of the disease, while others follow a more severe and potentially more debilitating course. Some of the more onerous secondary symptoms are calcinosis, vasculitic ulcers and contractures.

Genesis of the Disease
The immune system is a group of cells that normally protects the body from infection. In autoimmune diseases like JM, however, once these cells turn on their infection-fighting process, they cannot turn it off. This process therefore damages the body instead of protecting it.

One way the immune system cells fight infection is through inflammation. But when the cells cannot turn off the inflammation process, tissues are damaged. In JM sufferers, the skin rash and weak muscles are caused by vasculitis, an inflammation in the blood vessels that lie under the skin and in the muscles. Since blood vessels run throughout the human body, JM can also affect other systems such as the digestive tract.

What causes JM?
Many researchers believe that there is a genetic predisposition to autoimmune diseases. As such, they feel that a child afflicted with JM is likely to have a blood relative who suffers from another autoimmune disease such as diabetes or arthritis.

The autoimmune disease initially presents itself when an invasive "trigger" causes the body's immune system to overreact. It is believed that this trigger could be a virus, a vaccine or an environmental hazard.

What are the symptoms of JM?
The primary symptoms of JM are weak or painful muscles, skin rash (with JDM), fatigue and fever. Some children experience joint pain as well.

Weak muscles
The muscles that are affected the most are near the trunk of the body: the stomach, quadriceps, neck and biceps. However, other muscles can become weak as well. Sometimes there is inflammation in the esophagus which makes it difficult for the child to swallow. Other times, there is inflammation in the gastrointestinal tract, which causes bowel difficulties.
A child with JM has great difficulty climbing stairs and getting up from the floor or from a chair. Walking and running become very challenging and exhausting.
Skin rash
The skin rash in JDM usually occurs on the face, knuckles, elbows, knees and ankles. The rash may appear before, after or at the same time as the muscle weakness. Sometimes the rash is so faint that it is not noticeable.
The rash appears as a red purplish color on the eyelids and cheeks; whereas the rash looks like red dry skin patches over the knuckles, elbows and knees. The fingernails and the nailbeds may take on a pinkish color as well.
Sometimes, the children develop Gottron’s Papules, which are small whitish bumps that appear on the fingers. They look like small blisters.
Fatigue
With JM, the child becomes tired easily and can only walk short distances. The child needs to rest often and lacks the energy for normal activities. It becomes difficult for the child to keep up with friends.
Fever
Oftentimes, the child with JM runs low-grade fevers, especially at night. There has also been a connection discovered between a virus with high fevers just before the onset of JM.

What are other possible symptoms?
The following symptoms of JM are less common, but important to understand:

Calcinosis
About a third of the children with JM will develop calcinosis, which are small lumps of calcium that form under the skin or in the muscle. They may feel like little rocks under the skin and can range in size from a dot to a large pebble.
The lumps can either break through the skin and form a bump that may leak creamy white calcium, or they can be absorbed back into the body. If they break the skin, they can become infected and painful, and sometimes need to be surgically removed. They usually occur on the joints, such as the elbows and knees, but they can occur anywhere on the body.
Vasculitic Ulcers
Vasculitic ulcers are holes in the tissue that surrounds an inflamed blood vessel. They can occur in the skin or in the bowels (the organs that transport food from the esophagus to the rectum).
When they occur in the skin, they look like sores in the skin rash. They can be very deep or on top of the skin and they also range in size. The ulcers can be very painful, but will usually go away with treatment.
When the ulcers occur in the bowels, it is due to inflamed blood vessels in the protective lining around the bowels. They cause a break in the lining which can eventually harm the digestive organs.
The warning signs of this condition are severe stomach pain, dark black stools or blood in the stools, or a change in the pattern of bowel movements.
Contractures
Contractures are shortened muscles that cause a joint to stay in a bent position. These can occur due to lack of exercise or from scarring of the muscles.

What tests are used to diagnose JM?
If JM is suspected, blood tests are the first step to confirm that there is a problem. Muscle enzymes are measured, including CPK and aldolase, which are enzymes that leak from inflamed or damaged muscles into the bloodstream. Antinuclear antibodies (ANA) are also measured to see if the child’s body is producing antibodies against its own cells.

The next step is usually an MRI which can also detect muscle damage and inflammation. If the MRI shows evidence of diseased muscles, a muscle biopsy is typically performed to finalize the diagnosis. A small amount of muscle is removed for examination to determine if and how much the muscles and blood vessels have been affected by the disease.

What is the treatment for JM?
There is no cure for JM yet, but there are treatment options to help manage the symptoms. Early and aggressive treatment is usually the best predictor of a better outcome of this disease.

IV corticosteroids (Solumedrol) are usually the first line of treatment for JM. This is oftentimes coupled with high dose oral Prednisone, another corticosteroid. Since the side effects of corticosteroids can be very troublesome, Methotrexate (a chemotherapy drug when given in higher doses) is usually introduced early to allow for tapering of the corticosteroids.

Other common treatments include Cyclosporine and Intravenous Immunoglobulin (IVIG). Less common treatments, but still used include Cellcept (chemotherapy), Enbrel and Remicade.

These medications all have their own side effects, but the most common ones for Prednisone are: -increased appetite and weight gain -rounded face -mood changes -high blood pressure -stretch marks -fragile bones and bone damage -cataracts -slow growth

What is the prognosis?
It is believed that a third of the children will suffer from JM most of their life, while another third will experience temporary remissions and the other third will experience a permanent remission (no medications, no symptoms).

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